Case presentation
Initial evaluation
A 76-year-old man visited his family physician at the end of April X
(year) due to visual disturbance in the left eye and was referred to our
department on May 27, X. Ophthalmological history included bilateral
cataract surgery, left ptosis (both in X-4 years), intrascleral fixation
of left intraocular lens dislocation (in X-1 year), Irvine Gass
syndrome, and ocular hypertension. Detailed history-taking revealed that
the patient received treatment for IgG4-related disease (retroperitoneal
fibrosis; prednisolone 2.5 mg/day) from his primary care physician.
Current medical history
At the initial visit on June 2 X, there was no conjunctival hyperemia in
both conjunctivae, and anterior chamber cells were mildly present on the
left eye. The left optic nerve papilla was mildly erythematous and
swollen. Visual acuities were 1.2 (1.2 × S +1.75D) in the right eye and
0.6 (0.7 × S +2.00D) in the left eye, and intraocular pressures were 18
mmHg in the right eye and 16 mmHg in the left eye. Critical flicker
frequency (CFF) was decreased in the left eye (right ↑38Hz↓41Hz, left
↑36Hz↓36Hz), and Humphrey visual field test revealed inferior
auriculotemporal one-quarter blindness in the left eye (Figure 1). Brain
magnetic resonance imaging (MRI) showed no abnormal signal in the
infraorbital optic nerve; however, marked pituitary/pituitary
sclerenchyma enlargement with associated optic chiasm compression was
observed (Figure 2).
Intervention description/results
Based on the history and initial evaluation findings, a diagnosis of
left optic neuropathy associated with pituitary/pituitary scrofula
enlargement caused by IgG4-related disease was made. On June 10, visual
acuity and visual field defects improved (Figure 3), and upon
examination on July 7, the pituitary gland and pituitary pattern were
almost normalized (Figure 4), and the CFF decreased (right ↑42Hz↓43Hz,
left ↑37Hz↓43Hz) (39 Hz). The steroid dose was gradually decreased, and
maintenance therapy with 2.5-mg prednisolone daily was resumed on August
11.